Horner Syndrome Clinical Presentation

  • Author: Christopher M Bardorf, MD, MS; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 15, 2012
 

History

Obtaining a careful history is very helpful in the localization of lesions causing Horner syndrome.

  • First-order neuron lesions may be associated with signs and symptoms such as hemisensory loss, dysarthria, dysphagia, ataxia, vertigo, and nystagmus.
  • Second-order neuron lesions may be preceded by trauma and may be accompanied by facial, neck, axillary, shoulder or arm pain, cough, hemoptysis, history of thoracic or neck surgery, history of chest tube or central venous catheter placement, or neck swelling.
  • Symptoms associated with third-order neuron lesions include diplopia from sixth nerve palsy, numbness in the distribution of the first or second division of the trigeminal nerve, and pain.
  • The presence, absence, and/or location of anhydrosis is an important localizing sign that may be elicited from the history.
  • Although Horner syndrome is commonly an incidental finding related to a benign cause, it occasionally may be a manifestation of a serious and life-threatening disorder. Careful direction of the history to rule out such life-threatening disorders is of the utmost importance (see Causes).
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Physical

  • Important aspects of the physical examination include the following:
    • Measurement of pupillary diameter in dim and bright light and their reactivity to light and accommodation
    • Examination of the upper lids for ptosis
    • Examination of the lower lids for upside-down ptosis (eg, position of the lower lid with respect to the inferior limbus)
    • Extraocular movements
    • Biomicroscopic examination of the pupillary margin and iris structure and color
    • Confrontational visual field testing and testing of facial sensation
    • Observation for the presence of nystagmus, facial swelling, lymphadenopathy, or vesicular eruptions
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Causes

  • First-order neuron lesions
    • Arnold-Chiari malformation
    • Basal meningitis (eg, syphilis)
    • Basal skull tumors
    • Cerebral vascular accident (CVA)/Wallenberg syndrome (lateral medullary syndrome)
    • Demyelinating disease (eg, multiple sclerosis)
    • Intrapontine hemorrhage
    • Neck trauma (eg, traumatic dislocation of cervical vertebrae, traumatic dissection of the vertebral artery)
    • Pituitary tumor
    • Syringomyelia
  • Second-order neuron lesions
    • Pancoast tumor (tumor in the apex of the lung - most commonly squamous cell carcinoma)
    • Birth trauma with injury to lower brachial plexus
    • Cervical rib
    • Aneurysm/dissection of aorta
    • Subclavian or common carotid artery
    • Central venous catheterization
    • Trauma/surgical injury (eg, radical neck dissection, thyroidectomy, carotid angiography, coronary artery bypass graft)
    • Chest tubes
    • Lymphadenopathy (eg, Hodgkin disease, leukemia, tuberculosis, mediastinal tumors)
    • Mandibular tooth abscess
    • Lesions of the middle ear (eg, acute otitis media)
    • Neuroblastoma[2]
  • Third-order neuron lesions
    • Internal carotid artery dissection (associated with sudden ipsilateral face and/or neck pain)[3]
    • Raeder syndrome (paratrigeminal syndrome) - Oculosympathetic paresis and ipsilateral facial pain with variable involvement of the trigeminal and oculomotor nerves
    • Carotid cavernous fistula
    • Cluster/migraine headaches
    • Herpes zoster
  • Drugs (may cause symptoms similar to Horner syndrome and may affect any region)
    • Acetophenazine
    • Alseroxylon
    • Bupivacaine
    • Butaperazine
    • Carphenazine
    • Chloroprocaine
    • Chlorpromazine
    • Deserpidine
    • Diacetylmorphine
    • Diethazine
    • Ethopropazine
    • Etidocaine
    • Fluphenazine
    • Guanethidine
    • Influenza virus vaccine
    • Levodopa
    • Lidocaine
    • Mepivacaine
    • Mesoridazine
    • Methdilazine
    • Methotrimeprazine
    • Oral contraceptives
    • Perazine
    • Prilocaine
    • Procaine
    • Prochlorperazine
    • Promazine
    • Promethazine
    • Propoxycaine
    • Reserpine
    • Thioproperazine
    • Thioridazine
    • Trifluoperazine
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Contributor Information and Disclosures
Author

Christopher M Bardorf, MD, MS  Ophthalmologist, Children's Eye Physicians

Christopher M Bardorf, MD, MS is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Gregory Van Stavern, MD  Attending Physician, Department of Ophthalmology and Neurology, Washington University School of Medicine

Gregory Van Stavern, MD is a member of the following medical societies: American Academy of Neurology and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Enrique Garcia-Valenzuela, MD, PhD  Clinical Assistant Professor, Department of Ophthalmology, University of Illinois Eye and Ear Infirmary; Consulting Staff, Vitreo-Retinal Surgery, Midwest Retina Consultants, SC, Parkside Center

Enrique Garcia-Valenzuela, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, Retina Society, and Society for Neuroscience

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Reede DL, Garcon E, Smoker WR, Kardon R. Horner's syndrome: clinical and radiographic evaluation. Neuroimaging Clin N Am. May 2008;18(2):369-85, xi. [Medline].

  2. Smith SJ, Diehl N, Leavitt JA, Mohney BG. Incidence of pediatric Horner syndrome and the risk of neuroblastoma: a population-based study. Arch Ophthalmol. Mar 2010;128(3):324-9. [Medline].

  3. Pirouzian A, Holz HA, Ip KC, Sudesh R. Acquired infantile Horner syndrome and spontaneous internal carotid artery dissection: a case report and review of literature. J AAPOS. Apr 2010;14(2):172-4. [Medline].

  4. Almog Y, Gepstein R, Kesler A. Diagnostic value of imaging in horner syndrome in adults. J Neuroophthalmol. Mar 2010;30(1):7-11. [Medline].

  5. Mughal M, Longmuir R. Current pharmacologic testing for Horner syndrome. Curr Neurol Neurosci Rep. Sep 2009;9(5):384-9. [Medline].

  6. Perez-Inigo MA, Gonzalez I, Fernandez FJ, Díaz S, Ferrer C, Alias E, et al. [Usefulness of apraclonidine in the diagnosis of Horner syndrome]. Arch Soc Esp Oftalmol. Feb 2009;84(2):105-8. [Medline].

  7. Freedman KA, Brown SM. Topical apraclonidine in the diagnosis of suspected Horner syndrome. J Neuroophthalmol. Jun 2005;25(2):83-5. [Medline].

  8. Dewan MA, Harrison AR, Lee MS. False-negative apraclonidine testing in acute Horner syndrome. Can J Ophthalmol. Feb 2009;44(1):109-10. [Medline].

  9. Watts P, Satterfield D, Lim MK. Adverse effects of apraclonidine used in the diagnosis of Horner syndrome in infants. J AAPOS. Jun 2007;11(3):282-3. [Medline].

  10. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. 1994;4:2473-4.

  11. American Academy of Ophthalmology. Basic and Clinical Science Course: Neuro-ophthalmology. 1999-2000;5:97-99, 109-111.

  12. Fraunfelder, FT. Drug-Induced Ocular Side Effects and Drug Interactions. 1996;4:553.

  13. Gutman I, Levartovski S, Goldhammer Y, et al. Sixth nerve palsy and unilateral Horner's syndrome. Ophthalmology. Jul 1986;93(7):913-6. [Medline].

  14. Loewenfeld I. The Pupil: Anatomy, Physiology and Clinical Applications. 1993;2:1131-1177.

  15. Mokhtari F, Massin P, Paques M, et al. Central retinal artery occlusion associated with head or neck pain revealing spontaneous internal carotid artery dissection. Am J Ophthalmol. Jan 2000;129(1):108-9. [Medline].

  16. Roy FH. Ocular Syndromes and Systemic Diseases. 1989.

 
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